Giant cell arteritis (GCA) is a primary large-vessel vasculitis predominantly seen in the elderly that preferentially involves the external carotid artery and its branches. However, inflammation of the aorta and its branches occurs in a subset of patients although symptoms of aortic involvement may appear years after the initial diagnosis of GCA. Therefore, aortic involvement has probably been underestimated and its incidence may be more frequent than suspected. Systematic evaluation of patients with imaging techniques such as magnetic resonance imaging angiography (MRA) and positron emission tomography (PET) may reveal that the clinical impact of extracranial involvement by GCA may be more relevant than previously thought. Regarding the histopathology, there are some similarities between chronic periaortitis (including idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms and perianeurysmal retroperitoneal fibrosis), idiopathic aortitis, and GCA, suggesting that all these illnesses probably share common pathological mechanisms. Inflammatory aortitis can arise in different clinical settings been idiopathic aortitis more frequent than expected in surgical specimens of aortic aneurysm surgeries in the general population. In the setting of GCA an early diagnosis of aortic involvement is mandatory in order to perform a treatment capable of avoiding the chronic and acute complications associated with an elevated mortality [1, 2].